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systemic necrotizing vasculitis

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https://www.readbyqxmd.com/read/29625716/-ocular-manifestations-in-beh%C3%A3-et-s-disease
#1
A-C Desbois, C Terrada, P Cacoub, B Bodaghi, D Saadoun
Ocular disease in Behçet's disease is frequent and may be associated with a poor functional prognosis. Uveitis is the most common ocular manifestation in Behçet's disease and represents a diagnostic criterion of the disease. The ocular involvement is inaugural of the disease in 20% of the cases or may develop 2 to 3 years after the beginning of the extraocular signs. The risk of blindness at 5 years is in the order of 15 to 25%, mainly due to macular involvement or retinal vasculitis. Uveitis may be anterior, intermediate, posterior or panuveitis...
April 3, 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29558354/-the-changing-face-of-medium-sized-vasculitis
#2
Jolanta Parada-Turska, Monika Turska
Polyarteritis nodosa is a systemic necrotizing vasculitis which predominantly affects medium-sized arteries. It is a rare disease nowadays. Both the nomenclature and the classification of polyarteritis nodosa was amended several times in the past. Currently, there is a distinction between the primary form described as classical polyarteritis nodosa and other forms that are associated with their probable cause e.g. with viral hepatitis B, C or HIV infection. Moreover, polyarteritis-like necrotizing vasculitis can appear in the course of genetic diseases caused by mutations in single genes...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29541857/decidual-granulomatous-reaction-in-a-placenta-from-a-preeclamptic-pregnancy-a-case-report-and-review-of-the-literature
#3
Simon B Chen, Satoru Kudose, Hannah R Krigman
We report a case of decidual perivascular non-necrotizing granulomas in a placenta from a pregnancy complicated by severe preeclampsia with no evidence of infection. The mother was a 20-year-old primigravida with severe preeclampsia diagnosed in the third trimester with subsequent delivery of a healthy baby boy at 37 weeks 5 days gestation. Pathologic examination of the placenta showed scattered non-necrotizing granulomas in decidua, often adjacent to remodeled decidual arteries without fibrinoid necrosis...
March 14, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29501300/granulomatosis-with-polyangiitis-presenting-as-facial-nerve-palsy-in-a-teenager
#4
James C Wang, Brittany A Leader, Ryan A Crane, Bernadette L Koch, Matthew M Smith, Stacey L Ishman
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e...
April 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29496270/-scleritis-and-systemic-diseases-what-should-know-the-internist
#5
P Bielefeld, D Saadoun, E Héron, S Abad, H Devilliers, C Deschasse, S Trad, D Sène, G Kaplanski, P Sève
Scleritis is an inflammatory disease of the sclera; outer tunic of the eye on which the oculomotor muscles are inserted. It can be associated with a systemic disease up to one time out of 3. These associated diseases are mainly rheumatoid arthritis, vasculitis, including granulomatosis with polyangiitis in the first line and spondyloarthropathies. Before mentioning such an etiology, it is necessary to eliminate an infectious cause, mainly herpetic, which is regularly underestimated. The classification of scleritis is clinical...
February 26, 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29392482/ixazomib-induced-cutaneous-necrotizing-vasculitis
#6
A Alloo, H Khosravi, S R Granter, S M Jadeja, P G Richardson, J J Castillo, N R LeBoeuf
Ixazomib is a second-generation proteasome inhibitor that has been approved in the combination treatment of multiple myeloma and is currently under clinical investigation for the management of Waldenstrom's macroglobulinemia. While cutaneous adverse events secondary to proteasome inhibitors have been reported, the side effect profile of ixazomib remains to be documented. We report two patients, one with multiple myeloma and one with Waldenstrom's macroglobulinemia, who developed cutaneous necrotizing vasculitis after the initiation of ixazomib...
February 1, 2018: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/29368866/cutaneous-and-systemic-vasculitides-in-dermatology-a-histological-perspective
#7
Silvia Alberti-Violetti, Emilio Berti, Angelo V Marzano
Cutaneous vasculitides encompass a wide and heterogeneous group of diseases affecting skin blood vessels that are clinically characterized by polymorphic skin lesions, particularly including palpable purpura as well as urticarial and necrotic-ulcerative lesions, with possible, albeit rare, extracutaneous involvement. Cutaneous leukocytoclastic angiitis and urticarial vasculitis, which are the two prototypic and most common variants of this group, are usually idiopathic but may also be induced by different triggers, notably drugs and infections, or may manifest in association with systemic disorders, particularly lupus erythematosus...
April 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29302827/colonic-perforation-due-to-severe-cytomegalovirus-disease-in-granulomatosis-with-polyangiitis-after-immunosuppression
#8
REVIEW
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29282399/chronic-retinal-necrosis-severely-complicated-by-neovascular-glaucoma-a-case-report
#9
Takanori Matsuoka, Kazunobu Asao, Noriyasu Hashida, Kohji Nishida
Background: Chronic retinal necrosis (CRN) is a rare chronic granular necrotizing retinitis that was first described in 2013. CRN is characterized by intraocular inflammation accompanied by occlusive vasculitis, granular retinitis, and slowly progressing necrosis around the retina in a host with partial immune dysfunction. Cytomegalovirus (CMV) is reported to be a causative agent. There are several ocular complications such as retinal detachment and neovascular glaucoma; however, there has been no description of a clinical manifestation of neovascular glaucoma in CRN...
September 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/29260748/-necrotizing-sarcoid-granulomatosis-with-clinical-presentations-of-recurrent-acute-abdomen-case-report-and-literature-review
#10
REVIEW
V I Vasilyev, S G Palshina, B D Chaltsev, S G Radenska-Lopovok, T N Safonova
The authors have described the world's first case of necrotizing sarcoid granulomatosis (NSG) in a 22-year-old woman with the clinical presentations of acute abdomen, which are associated with abdominal lymph nodal infiltration and necrosis, obvious constitutional disturbances (fever, nocturnal sweats, and significant weight loss), high inflammatory activity (anemia, leukocytosis, high erythrocyte sedimentation rates and C-reactive protein levels), the gradual appearance of splenic and hepatic necrotic foci, and infiltration into the lung and lacrimal glands with the development of unilateral uveitis...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#11
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29239918/anti-neutrophil-cytoplasmic-antibody-negative-central-nervous-system-granulomatosis-with-polyangiitis-and-its-clinical-characteristics
#12
Zhihua Chen, Yifeng Miao, Hui Wu, Ran Wang, Zhiyi Zhou, Shilei Zhang, Longtian Chen, Yongming Qiu
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions...
March 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29209584/sudden-death-from-spontaneous-coronary-artery-dissection-due-to-polyarteritis-nodosa
#13
Cyrus M Munguti, Paul M Ndunda, Tabitha M Muutu
Spontaneous coronary artery dissection (SCAD) is an emerging and rare cause of acute coronary syndrome and sudden cardiac death. While it was previously reported among young females with fibromuscular dysplasia, new literature indicates that this condition could occur in older populations. Polyarteritis nodosa (PAN) causes systemic necrotizing vasculitis which typically affects small to medium-sized muscular arteries and could affect the coronary arteries. A few case reports of PAN causing acute coronary artery disease have been reported in the literature...
October 2, 2017: Curēus
https://www.readbyqxmd.com/read/29189887/eosinophilic-granulomatosis-with-polyangiitis-without-respiratory-symptoms-or-asthma-in-an-adolescent-case-report-and-literature-review
#14
REVIEW
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#15
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29127573/eosinophilic-granulomatosis-with-polyangiitis-complicated-by-subarachnoid-hemorrhage-and-coronary-vasculitis-a-case-report-and-review-of-the-literature
#16
REVIEW
Shogo Matsuda, Shuzo Yoshida, Youhei Fujiki, Hidetoshi Satomi, Tohru Takeuchi, Yoshinobu Hirose, Shigeki Makino, Shigeki Arawaka
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0...
November 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#17
LETTER
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
No abstract text is available yet for this article.
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29044308/heart-conduction-system-defects-and-sustained-ventricular-tachycardia-complications-in-a-patient-with-granulomatosis-with-polyangiitis-a-case-report-and-literature-review
#18
Laryssa Passos Sarmento Santos, Victor Guerreiro Bomfim, Camila Fagundes Bezerra, Natália Vieira Costa, Rafael Barreto Paes de Carvalho, Ricardo Sobral de Carvalho, Rogério da Hora Passos, Olivia Carla Bomfim Boaventura, André Luiz Nunes Gobatto
Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances...
July 2017: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/29032100/-severity-of-illness-index-in-autoimmune-diseases-have-there-any-usefulness-in-medical-practice
#19
A Saunier, M-A Vandenhende, P Morlat, F Bonnet
INTRODUCTION: Assessing disease activity in patients suffering from autoimmune diseases is complex. Symptoms are multiple, often subjective and there are no reliable biomarkers. Many activity scores have been implemented to compare treatment efficacy in clinical trials. Their use in clinical practice is largely unknown. We performed a practical survey to analyze the use of activity scores in clinical practice to consider treatment response and to assess the determinants of their use. METHODS: A sample of French internists answered a questionnaire about activity scores of systemic lupus erythematosus, Sjögren's syndrome, autoimmune myositis and necrotizing vasculitis of small vessels...
November 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28992397/prognostic-significance-of-cavitary-lung-nodules-in-granulomatosis-with-polyangiitis-a-clinical-and-imaging-study-of-225-patients
#20
Bailey A Russell, Sindu Mohan, Rachandeep Chahal, Simon Carette, Christian Pagnoux
BACKGROUND: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis with pulmonary nodules as a common manifestation. Our study examined whether pulmonary nodules, and nodule type (solid versus cavitary), are associated with different disease manifestations and outcomes. METHODS: Demographic, clinical, biological, radiological data at diagnosis, during follow-up, and treatments of GPA patients followed at the Mount Sinai Hospital (Canada) vasculitis clinic were analyzed...
October 9, 2017: Arthritis Care & Research
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